Cutaneous T-Cell Lymphomas

National Organization for Rare Disorders, Inc.

ImportantIt is possible that the main title of the report Cutaneous T-Cell Lymphomas is not the name you expected.

Disorder Subdivisions

None

General Discussion

General Discussion

Summary

Cutaneous T-cell lymphomas (CTCLs) are a group of disorders characterized by abnormal accumulation of malignant T-cells in the skin potentially resulting in the development of rashes, plaques and tumors. CTCLs belong to a larger group of disorders known as non-Hodgkin's lymphomas (NHLs), which are related malignancies (cancers) that affect the lymphatic system (lymphomas).

Introduction

Functioning as part of the immune system, the lymphatic system helps to protect the body against infection and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid known as lymph from different areas of the body into the bloodstream. Lymph accumulates in the tiny spaces between tissue cells and contains proteins, fats, and certain white blood cells known as lymphocytes.

There are two main types of lymphocytes: B-lymphocytes, which may produce specific antibodies to "neutralize" certain invading microorganisms, and T-lymphocytes, which may directly destroy microorganisms or assist in the activities of other lymphocytes. CTCLs result from errors in the production of T-lymphocytes or transformation of T-lymphocytes into malignant cells. In CTCLs, abnormal, uncontrolled growth and multiplication (proliferation) of malignant T-lymphocytes result in accumulation of these lymphocytes in the skin. In some cases, malignant lymphocytes may spread to affect the lymph nodes and eventually to other bodily tissues and organs, potentially resulting in life-threatening complications. The specific symptoms and physical findings may vary from case to case, depending upon the extent and region(s) of involvement, the specific type of CTCL present, and various additional factors.

Non-Hodgkin's lymphomas, such as CTCLs, may also be categorized based upon certain characteristics of the cancer cells as seen under a microscope and how quickly they may tend to grow and spread. For example, CTCLs may be characterized as "low-grade" (or indolent) lymphomas, which tend to grow slowly and result in few associated symptoms, or "intermediate-grade" or "high-grade" (aggressive) lymphomas, which typically grow rapidly, requiring prompt treatment. Most cases of CTCL, especially the classic form (mycosis fungoides), are slow-growing (indolent) lymphomas.

Supporting Organizations

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For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders (NORD). For a full-text version of this report, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

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Last Updated: 10/25/2013Copyright 2013 National Organization for Rare Disorders, Inc.

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